[Chronic benign familial pemphigus. Clinical, histological and immunological study of 7 cases]

A clinical, histopathological and immunological study was carried on a series of seven patients of Familial Benign Chronic Pemphigus (FBCP). This condition is characterized by recurrent small blisters, mainly, on intertriginous areas and on the sides of the neck, that become wet and crusted rapidly. They are generally sharply marginated and Nikolsky's sign is often positive. The lesions appear spontaneously and may be precipitated by warm, humid environment, mechanical trauma, radiations, bacterial or mycotic infection. Healing occurs with residual non scarring hyperpigmentation. Histopathologically, the epidermal alteration respond to a primary acantholytic mechanism. Ultramicroscopic studies have suggested an alteration on the desmosome-tonofilament complex. Comparatively with Pemphigus, another acantholytic disease in which immunological pathogenesis is strongly suspected, only few reports are referred to immunological studies in FBCP. In the present paper, a direct immunofluorescent study on spontaneous and provoked blisters was made in order to investigate deposits of immunoglobulins and complement. Indirect IF was performed with sera of teh patients for detection of circulating antibodies. Two cases were also sensitized with erythrocytic antigen. The immunological response to this substance was evaluated. The clinical and histopathological findings of the present series, are similar with previous descriptions. The immunofluorescent studies do not provide evidence of antibodies to epidermal intercellular space, like Pemphigus.(ABSTRACT TRUNCATED AT 250 WORDS)